Ординатура / Офтальмология / Английские материалы / Primary Care Ophthalmology_Palay, Krachmer_2005

FIGURE 13–19 Home massage over the nasolacrimal sac to open obstruction. This maneuver should be done three to four times a day, followed by instillation of an antibiotic drop such as polymyxin B/trimethoprim (Polytrim). If the obstruction does not resolve after 2 to 3 weeks and discharge is still present, referral for probing is indicated.

FIGURE 13–20 Dacryocele. These lesions usually are congenital and must be treated immediately by an ophthalmologist.

•After the age of 6 months, obstructions are far less likely to resolve, and the child should be referred to an ophthalmologist.

•With mucopurulent discharge, topical antibiotics (e.g., polymyxin B/trimethoprim [Polytrim] 1 drop 3 to 4 times a day) are administered for 1 to 3 weeks with massage and frequent cleansing of secretions. If the bacterial infection resolves, the system may open spontaneously.

•With a mass such as mucocele or dacryocele, rapid referral to an ophthalmologist is necessary (Fig. 13–20).

•If antibiotic drops do not clear the mucopurulent discharge in 1 to 2 weeks, the patient should be referred to an ophthalmologist, regardless of age. These children have severe obstructions with bacterial colonization, and the condition will rarely resolve unless probing is done. Constant bacterial infection in the lacrimal sac increases the risk of preseptal cellulitis.

•Nasolacrimal duct probing is 95% effective in opening obstructions if performed before 1 year of age. If performed before the child is 2 years old, probing is approximately 85% successful. Because spontaneous opening is unlikely after 6 months of age or in the presence of infection, and this procedure has a higher rate of success in some studies if done early, the treatment plan must be adjusted accordingly.

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Probing can be performed in the ophthalmologist’s office without anesthesia if the child is younger than 3 or 4 months of age. After this age, anesthesia is almost always necessary.

•Silastic tubing can be placed to keep the duct system open in older children and those in whom probing fails to open the duct.

•A balloon catheter procedure to widen the system may improve success in recurrent cases.

•Marsupialization of associated intranasal cysts may be necessary with mucoceles.

•Surgical creation of a passage between the tear lake, nasolacrimal sac, and nasal mucosa (dacryocystorhinostomy, or Jones tube placement) is reserved for older children or those in whom probing and Silastic tube placement do not resolve the problem. Such procedures are much more invasive and often can be avoided if early treatment is instituted.

Retinopathy of Prematurity

Symptoms

• No symptoms are present.

Signs

•The disorder is almost always bilateral but often asymmetrical.

•Pupils are small and immobile in the late stages, and iris blood vessels are enlarged.

•Most signs are seen only with the indirect ophthalmoscope. A ridge of abnormal vascular tissue grows from the retina into the vitreous. Blood vessels may become dilated and tortuous.

• If advanced stages are not treated, retinal detachment and blindness are likely. Leukocoria also may result if the detached retina comes to lie behind the lens.

•As children get older, strabismus (either esotropia or exotropia) can be seen as a late sequela.

•Microphthalmia (a small eye) and phthisis (a shrunken eye) can result after retinal detachment.

Associated Factors and Diseases

•Low birth weight, young gestational age, sepsis, and perinatal oxygen administration are risk factors for the disorder.

•All babies born at 32 weeks of gestation or before, weighing less than 1500 g, or with an unstable clinical course should be screened by an ophthalmologist familiar with retinopathy of prematurity at 31 weeks of postconceptional age or 4 weeks of postnatal age, whichever is later. If no retinopathy of prematurity is present, or the disorder resolves, these children still require follow-up with an ophthalmologist at approximately 3 months after discharge and throughout the first few years of life because of their increased risks of strabismus, high refractive error (i.e., need for glasses), amblyopia, optic atrophy, and retinal abnormalities.

Treatment

•Once the treatment threshold is reached, cryotherapy or laser therapy of the retina must be performed, usually within 72 hours. An ophthalmologist trained in management of retinopathy of prematurity makes the determination of threshold disease based on strict criteria. A 2003 study found benefit in treating earlier than the previous threshold designation from the original CRYO-ROP study. Another 2003 study found that strictly monitoring and limiting the oxygen babies receive in the neonatal care unit also may reduce the rate of severe retinopathy of prematurity. Future studies will better delineate the optimal amount of oxygen for premature babies. Up to 95% of infants with threshold disease who receive laser therapy retain vision, whereas before modern treatments were available, a majority developed blindness as a result of retinal detachment or scarring.

•Throughout life, glasses, patching, surgery, and other modalities may be needed to treat the sequelae of retinopathy of prematurity.

Congenital Anomalies

Intrauterine Infections

Infections that cross the placenta, such as toxoplasmosis, rubella, and infections due to cytomegalovirus, herpes virus, varicella virus, lymphocytic choriomeningitis virus (LCMV), and human immunodeficiency virus (HIV), often cause characteristic eye conditions. If an intrauterine infection is suspected, an ophthalmology consultation may help confirm the diagnosis. Vision often is affected, so early referral is essential.

Congenital Cataracts

Symptoms

•Vision is mildly to severely decreased.

•In severe cases in which essentially no vision is present, the infant may keep the eyes closed.

•When the condition results in a partial cataract, the infant may squint, especially in bright sunlight, to decrease the resultant glare.

Signs

•Leukocoria (white pupil) is present if the cataract is dense (Fig. 13–21).

•The red reflex is abnormal or absent.

•If the condition developed within the first 3 months of life, nystagmus may be present.

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FIGURE 13–21 Leukocoria. This child has a unilateral cataract. Leukocoria is seen as a white pupil when viewed with a penlight or in room light.

Differential Diagnosis

Diagnostic possibilities include the following:

•Cataract

•Retinoblastoma

•Coats’ disease (an exudative retinal detachment caused by vascular anomalies)

•Retinopathy of prematurity

•Chorioretinal coloboma (white sclera where retina should be)

•Corneal scar (Peters’ anomaly)

•Retinal detachment

Associated Diseases

•Many genetic and metabolic disorders are associated with congenital cataracts. Some respond to dietary or other treatment. All infants with early-onset cataracts should have a genetic and/or metabolic evaluation.

Treatment

•Congenital cataract constitutes an emergency. The brain learns to see with the macula (the center of the retina where 20/20 vision is possible) most rapidly during

the first 3 to 4 months of life. If vision is severely limited during this critical period of visual development, it cannot be restored completely. Thus, congenital cataracts must be surgically removed, and optical rehabilitation with contact lenses, intraocular lenses, or glasses must be in place before 3 to 4 months of age. Many patients can have near-normal vision if they receive prompt treatment. With treatment after this age, some vision may be restored, but the result often is in the 20/200 or legally blind range.

•An ophthalmologist determines the adequacy of the infant’s visual stimulation in cases of partial cataract.

•Persistent hyperplastic primary vitreous, also called persistent fetal vasculature, is a type of congenital cataract that may result in a small eye and painful glaucoma.

•All infants should be screened for a red reflex before discharge from the nursery and at each well-baby checkup. If a cataract or other disruption of the red reflex is suspected, referral to an ophthalmologist is needed before the infant is 3 months old.

Congenital Ptosis

The lid is elevated primarily by the levator muscle, which inserts onto the tarsal plate of the eyelid, and by the orbicularis oculi muscle, which encircles the eye. The levator is innervated by the third cranial nerve. The lid also is partially elevated by Müller’s muscle, which is innervated by sympathetic fibers. These fibers travel from the brain out through the spine, around the neck, over the lung, and then up around the carotid artery to innervate the pupil and muscle of the eyelid.

Symptoms

•If the ptosis is severe, vision is greatly decreased.

•If the condition is bilateral, the infant never opens the eyes or never focuses.

Signs, Etiology, and Differential Diagnosis

•A faintly defined or no lid crease may signal an abnormal insertion of the levator aponeurosis or absence of the entire muscle or insertion. Lack of a lid crease with normal function is common in Asian patients.

•One or both brows are often markedly elevated.

•The infant may keep a chin-up position (Fig. 13–22).

•Mild ptosis with a lid crease, a small pupil (miosis), a difference in iris color (heterochromia), and an inability to sweat (anhidrosis) on one side of the face are signs of Horner syndrome caused by sympathetic nerve interruption. In congenital cases, a history of brachial plexus injury or difficult delivery may help determine the etiology. If the history is not suggestive, workup for neuroblastoma and other anatomic abnormalities is indicated.

•If the lid is thickened or red, a hemangioma may be present.

Treatment

•Surgical correction often is necessary.

•If the ptosis is untreated, amblyopia and loss of vision often result. Ophthalmologic referral as soon after birth as possible is indicated.

FIGURE 13–22 Congenital ptosis. Baby keeps the chin up to see. Note elevated brows and lack of right lid crease.

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•If the condition is severe, surgical correction using a tarsal sling must be performed before the infant is 3 months of age.

•Often ptosis induces refractive error (e.g., myopia, astigmatism), which must be treated with glasses.

•Amblyopia may be severe and requires treatment with patching; if ptosis is asymmetrical, amblyopia is best treated before the infant is 3 months of age.

Congenital Glaucoma

Symptoms

•Vision is decreased.

•Photophobia is characteristic.

Signs

•Excess tearing occurs, especially during daylight or in bright light.

•The corneas are enlarged.

•Hazy or cloudy corneas cause a diminished corneal reflex.

Differential Diagnosis

•Nasolacrimal duct obstruction: Congenital glaucoma often is associated with blindness, whereas nasolacrimal obstruction generally is benign. Nasolacrimal duct obstruction often results in mucopurulent discharge and excess tearing over time. Even if no mucopurulent discharge is present on external examination, if gentle pressure is exerted on the nasolacrimal sac with a cotton-tipped swab, discharge often can be expressed. If this sign is not present and excess tearing continues for more

than a few weeks, congenital glaucoma may be present. Simple nasolacrimal duct obstruction is not associated with photophobia; that is, in normal lighting, the infant should have the eyes open and be looking around the room. If the infant is reluctant to do this and keeps the eyes closed much of the time, either severe nasolacrimal duct obstruction with bacterial superinfection may be present or congenital glaucoma may be present. Simple nasolacrimal duct obstruction should not be associated with any cloudiness or abnormal appearance of the eyes. Congenital glaucoma may be associated with other congenital anomalies, especially Turner syndrome, Down syndrome, Rubinstein-Taybi syndrome, and Lowe syndrome.

Treatment

•Patients should be referred to an ophthalmologist.

•Medications, usually systemic or topical carbonic anhydrase inhibitors and topical beta blockers, may be used for short-term management to relieve pressure; however, they are rarely sufficient to preserve vision.

•Surgery is almost always needed. The approach to glaucoma, and the choice of procedures, are quite different in children than in adults, and consultation with a pediatric ophthalmologist or a glaucoma specialist familiar with glaucoma in children is

250 CHAPTER 13 • Pediatric Ophthalmology

because of the known association of this condition with severe amblyopia in the affected eye.

•Referral to an ophthalmologist is necessary at any age if the hemangioma enlarges, causes ptosis, or occludes the pupil.

•If vision is the same in both eyes and little or no induced astigmatism or myopia is present, observation usually is sufficient.

•In most cases, the induced refractive error differs between the eyes. Glasses are prescribed, and patching may be warranted to treat the amblyopia.

•If the hemangioma is on the upper lid or in another area in which it markedly interferes with vision and it is enlarging, it constitutes an ophthalmologic emergency. A course of systemic corticosteroids, usually prednisone at 2 mg/kg per day for up to 1 or 2 weeks, with a very slow taper thereafter, often achieves excellent results. Intralesional injection of corticosteroids also can be very effective, but severe complications such as central retinal artery occlusion have rarely been reported. Endocrinology referral may be advised to follow infants on steroids.

Follow-up

•Patients must be monitored by a pediatric ophthalmologist and a pediatrician.

•Long-term follow-up care is required until the capillary hemangioma regresses.

Port-Wine Stain

Symptoms

• No symptoms exist.

Signs

•A large, flat, purplish lesion is found on the skin of the face or scalp. Such lesions also may be present on other parts of the body.

•Glaucoma is likely to occur on the affected side, especially if the upper lid is involved.

•Seizures and developmental delay often are noted as well. When intracranial involvement of the hemangioma occurs with these signs, Sturge-Weber syndrome is diagnosed.

Treatment

•Because ipsilateral glaucoma occurs in approximately 50% of cases of port-wine stain (nevus flammeus) when it affects the upper eyelid, all children with the disorder should be referred for pediatric ophthalmologic consultation as soon as the lesion is detected. Magnetic resonance imaging (MRI) scan, computed tomography (CT) scan of the brain, and an electroencephalogram also may be indicated.

•Laser eradication of the lesion in some cases is now possible, but this treatment’s effect on the incidence of glaucoma is not known. Generally, the laser treatment must be performed within the first 2 years of life to be successful; therefore, early referral is essential.

Congenital Esotropia and Exotropia

Variable, intermittent ocular misalignment is normal for the first 3 to 4 months of life; however, misalignment after 3 to 4 months of age is not normal. In addition, a constant misalignment of either or both eyes is always abnormal at any age. Children who have a constant deviation of one or both eyes should be referred to an ophthalmologist immediately. This finding may be a sign of a developmental anomaly or tumor in the eye. Congenital esotropia is a condition in which affected children have essentially normal eye movements for the first 3 to 4 months of life but exhibit crossing of the eyes before 6 months of age. Congenital exotropia is a condition in which one or both eyes deviate outward toward the ear.

Symptoms

•Early in the disease, double vision may occur.

•Decreased stereopsis (depth perception) is present throughout life.

Signs

•Strabismus, with one or both eyes turning in toward the nose (esotropia) or outward toward the ears (exotropia), is characteristic.

•Most patients with congenital esotropia are unable to abduct the eyes (look laterally) fully.

•Nystagmus occurs in a small subset of patients with congenital esotropia.

•A decreased ability to make eye contact and bond with parents is noted in some cases, which improves after treatment.

Differential Diagnosis

•Early-onset sixth nerve palsy or paresis may mimic congenital esotropia. This deficit often is caused by increased intracranial pressure and is associated with nausea, vomiting, lethargy, and increased head circumference.

•In premature infants, the development of esotropia may be related to neurologic causes: The disorder often is associated with periventricular leukomalacia and cerebral palsy. This type of esotropia may occur at the same age as for congenital esotropia, but the ocular misalignment varies throughout the examination.

•Congenital third nerve palsy is a possible cause of exotropia.

•If no other symptoms are present and the perinatal or birth history is normal, the condition can be an isolated finding in an otherwise healthy, normal infant.

Associated Factors and Diseases

•The risk of intracranial pathology is increased in congenital exotropia; therefore, neuroimaging often is indicated.

•Some children with severe esotropia have motor delays because they can never look straight ahead when they are holding the head straight. Their eyes are always pulled into the esotropic position. These children may turn the head to the side to attempt to crawl or walk and often gain developmental motor milestones rapidly after surgery.

252 CHAPTER 13 • Pediatric Ophthalmology

Treatment

•Any patient with a constant deviation at any age, or any deviation constant or intermittent after 3 to 4 months of age should be referred for ophthalmologic evaluation.

•For patients with amblyopia, treatment immediately after diagnosis is most effective and usually involves patching and/or glasses.

•Stereopsis, the type of depth perception that results from the use of both eyes, can best be achieved in children with congenital esotropia or exotropia if surgery is performed before the age of 2 years. Approximately 65% of children need one surgical procedure to straighten their vision; 35% may require a second procedure. Ideally, the eyes should be straightened, regardless of the number of surgical interventions needed, before the patient is 2 years of age, to increase the chance of acquiring stereopsis. The value of stereopsis is twofold: (1) It allows the normal visual experience for the child, and (2) if stereopsis is achieved, the brain may work to keep the eyes straight throughout life. In children who do not have stereopsis, even if the eyes initially are straight, the risk for development of recurrent strabismus is greatly increased.

•Immediate referral of patients for ophthalmologic and neurologic evaluation is needed if acute crossing (esotropia) is associated with nausea, vomiting, lethargy, increased head circumference, and sunsetting of the eyes. These are signs of increased intracranial pressure.

•Parents need to be educated about the long-term nature of their child’s treatment. Children with congenital strabismus often experience lifelong strabismus and stereopsis issues.

Nystagmus

Nystagmus may be a sign of a life-threatening neurologic disorder or blindness. Thus, any child who has nystagmus with the eyes in the primary position should be referred to a pediatric ophthalmologist.

Symptoms

•Vision is decreased.

•Vision in the dark may be decreased.

Signs

•A constant, jiggling movement of the eyes is evident. The eye movements may be horizontal, vertical, or rotary, or a combination of these.

•The disorder may be bilateral, unilateral, or asymmetrical.

•Compensatory head shaking or nodding is possible.

•Weight loss may occur.

•Failure to thrive may be associated with this disorder.

•Nystagmus may be present at birth but more commonly develops over the first few months of life.